Unilateral subependymal grey matter heterotopia

Case contributed by Mario Javier Ordoñez Franco
Diagnosis certain

Presentation

Chronic onset of headache. No seizures or any other neurological symptoms associated with the headache. Laboratory examinations normal.

Patient Data

Age: 10
Gender: Female

Non contrast cerebral CT shows two well defined nodular lesions located in the ependymal of the lateral wall of the occipitall horn of the left lateral ventricle, with density is similar to the grey matter.

The nodular lesion does not have positive mass effect and no hydrocephalus is associated.

Nodular and oval lesions located in ependymal-subependimal location in the left lateral ventricle, these lesions follow the same signal as the grey cortical matter on all sequences.

No hydrocephalus associated, no other intra or extraaxial node or mass.

The left caudate nucleus and thalamus are well depicted and not related to the nodular lesions.

Case Discussion

Subependymal grey matter heterotopia is a malformation of cortical development, where there is an abnormal neural migration of cells to the subependymal region, this is not a neoplastic proliferation of cells. The majority of cases are sporadic, some are X-linked and these females tend to develop epilepsy.

These types of malformations can be bilateral diffuse, unilateral diffuse or unilateral focal.

Typically these lesions follow the grey matter density and intensity with no calcifications; associated with anomalous ventricle; no mass effect and frequently seen in the trigones and occipital horns.

Differential diagnoses are:

The head of the caudate nucleus is the only grey matter that lines the ventricles.

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